Tumeur nerveuse géante du thorax au cours de la maladie de Von Recklinghausen

We report the case of a patient aged 23, admitted for bilateral intrathoracic tumor, including a giant right. Surgery was performed by right sternothoracotomy. After 7 days, she presented an irreversible cardiac arrest. The malignant peripheral nerve sheath tumors are rare and aggressive. Their incidence is 0.001% in the general population and 0.16% in patients with neurofibromatosis type 1. These tumors are characterized by their risk of recurrence and poor prognosis. The treatment is the surgical resection. We analyze incidence, diagnosis and prognosis of these tumors.